Neuromuscular Illnesses In Children

What assessments are done for children with Neuromuscular Disorders?

Health history

- Pregnancy history (was mom expose to anything that was bad)

- birth history (*prematurity, complications? Tramautic birth?)

- family history (genetics)

- Changes in gait, poor feeding, changes in muscle tone, poor development (FTT)

Physical exam :

- Assess for muscle tone (hypotonia, hypertonia/spasticity), atrophy, motor development, sensory function

- Assess development (*head control)

- Assess reflexes- note persistence of primitive reflexes

- Assess LOC

- Skin depressions, hair tuffs on spine (spina bifida)

What are common Labs and Diagnostic Tests of Children with Neuromuscular Disorders?

- X-rays (looking at bones)

- Myelography (looks at muscles)

- Ultrasound

- CT/MRI

- Muscle biopsy (taking muscle tissue)

- Creatine kinase (can be indictive of any muscle damage)

- Reflects muscle damage

- We see CK in muscle damage

- Enzyme that is release when there is muscle breakdown

What are common Nursing diagnosis for patients with Neuromuscular Disorders?

- Impaired physical mobility

- Imbalanced nutrition: less than body requirements

- Urinary retention

- Constipation

- Self-care deficit

- Risk for skin integrity

- Risk for injury

- Interrupted Family process

Common Medications for Neuromuscular Disorders?

- Benzodiazepines (diazepam, lorazepam)

- Anticonvulsant and relief of muscle spasms (calming)

- Baclofen -> muscle relaxer

- Corticosteroids

- Botulin Toxim -> botox

What are common Treatments for Neuromuscular Disorders?

- Physical therapy

- Orthotics (AFO's)

- Traction

What is Cerebral Palsy?

range of nonspecific clinical symptoms; abnormal motor pattern and postures; NONPROGRESSIVE abnormal brain function;

(Movement disorder in children; lifelong )

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Etiology: before, during, post delivery; often no specific cause can be identified

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(NOT progressive, not terminal)

What are assessment findings of Cerebral Palsy?

classified in 3 types

Spastic:

- Exaggerated reflexes

- persistence of primitive reflexes,

- hypertonic

- poor control of posture/balance

- clonus

- scissor legs

Dyskinetic:

- Limp/flaccid

- uncontrolled worm like movement

- affects all extremities and face

Ataxic:

- Wide based gait

- poor coordination,

- unsteady gait

What is the goal for patients with Cerebral Palsy?

Goal: Assist child to obtain optimal development and function

What are interventions used for Cerebral Palsy?

- multidisciplinary

- PT, OT, speech therapy

- Assistive devices (AFOs)

- Pharmacologic

- Manage spasticity

- Reduce drooling

- Treat seizures

- Surgical

- Tendon lengthening

- Hip/spine surgeries

- VP shunts if CP complicated with hydrocephalus

- Nutritional support

What education needs to be done for Cerebral Palsy?

Education:

- major family support

- refer to early interventions services (under age of three)

What are the 3 primary types of Neural tube defects?

3 primary types :

Spina bifida occulta (less severe)

- Give folic acid to close neural tubes in pregnant moms.

Meningocele

Myelomeningocele (more severe)

Etiology: not known but associated with drugs, malnutrition, chemicals, genetics;

folic acid (400mcg) supplementation can decreases incidence up to 70%

What is Spina bifida occulta?

defect of the vertebral bodies without protrusion of the spinal cord or meninges; not externally visible;

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10%-20% healthy people have without complications (dimple in spine or tuff of hair)

What are assessment findings of Spina bifida occulta?

- Usually benign and asymptomatic

- Dimpling, abnormal patches of hair, dislocation of skin in lumbrosacral area (or anywhere along spine)

- Complicated occulta "tethered cord"

- Back pain

- Difficulty with bowel/bladder

- Motor delays

What are interventions done for Spina bifida occulta?

- Usually none

- Complicated occulta may require surgical intervention

What is Spina bifida cystica?

visual sac protrusion; 2 types:

Meningocele:

- meninges, spinal fluid; usually no neurological deficits

Myelomeningocele

- meninges, spinal fluid, nerves; spinal cord usually ends at point of defect...loss absent motor and sensory...leading to:

- Paralysis

- orthopedics issues

- bowel/bladder incontinence;

high percentage of children with myelomeningocele have hydrocephalus; frequent latex exposure increase risk of latex allergy—treat as latex allegic; may or may not have learning problems; lifelong follow up and coordination of care

What are assessment findings of Spina bifida cystica?

Often identified in utero (ultrasound and increase in AFP by blood)

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- Visible external sac along the spine

- Flaccid paralysis and Absence of deep tendon reflexes

- Lack of pain perception in lower extremities

- Relaxed anal sphincter

- Dribbling of urine

What are initial care is done for Spina bifida cystica?

Initial care:

- Preventing trauma

- Cover with sterile soaked gauze; report any leakage from sac

- Position in prone position in warm isolette- no holding

- Frequent head circumference

- Preventing infection

- Keep area free of urine and feces with sac or incision after - repair

- Watch for s/s of meningitis

What are continued care is done for Spina bifida cystica?

Continued care

- Promoting urinary elimination- Neurogenic bladder

- Intermittent catheterization & medications to improve bladder function

- Prevention/identification of infection

- Frequent cath increase risk of infection/kidney damage

- Possible VP shunt increase risk

- Promoting bowel elimination

- Promoting adequate nutrition

- Maintaining skin

- Promote mobility/orthopedic conditions

What education needs to be done for Spina bifida cystica?

- Performing clean intermittent catheterization

- Perform bowel care/training

- Recognizing s/s of infection

- Promoting mobility

- Preventing skin breakdown

- Promoting parents/child is expert in care

- Refer to Spina bifida association/ support groups

- SUPPORT, LISTEN

What is Muscular Dystrophy?

Duchenne muscular dystrophy- progressive muscle weakness and wasting; fatal

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Etiology: X-linked

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(Most common: Duchenne)

What are assessments done for Muscular Dystrophy?

- Decrease walking, increase falling

- Pseudohypertrophy ---atrophy

- Gowers sign -> hallmark sign

- Muscle biopsy and Serum creatine kinase are elevated

What interventions are done for Muscular Dystrophy?

- Corticosteroids

- Calcium supplements

- Orthotics

- Mobility aids

- Surgical

- Cardiopulmonary interventions

What education needs to be done for Muscular Dystrophy?

- encourage activities, (bed rest is NOT an appropriate intervention)

- provide emotional support to child and family

- refer to MD association

What is Juvenile Idiopathic Arthritis?

autoimmune disorder; autoantibodies target joints; some forms may affect eyes;

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chronic disorder with relapse and remissions

What are assessment findings of Juvenile Idiopathic Arthritis?

Assessment:

- Irritable infant

- Poor weight gain/delayed growth

- Eye inflammation

- Fever (systemic disease)

- Rash (nonpruritic, macular rash)

- Pain, Redness, Warmth

- Stiffness (esp in the morning)

- Swelling

- Limping

Anemia and elevated ESR...may/rarely have a positive rheumatoid factor

What are interventions done for Juvenile Idiopathic Arthritis?

- inflammation control

- Pain relief

- Antirheumatic drugs (methotrexate; Enbrel)

- Regular eye exams -> it hurts the eyes

- ROM activities

- Splints for nighttime sleep; firm mattress

- Warm bath/compressions

Education: encourage a support group